Genomes and Genes
Gene Symbol: Pax3
Description: paired box 3
Alias: Pax-3, Splchl2, splotch, paired box protein Pax-3, PAX3/FKHR fusion, paired box gene 3
Publications155 found, 100 shown here
- Lbx1 specifies somatosensory association interneurons in the dorsal spinal cordMichael K Gross
Molecular Neurobiology Laboratory, The Salk Institute, 10010 North Torrey Pines Road, La Jolla, CA 92037, USA
Neuron 34:535-49. 2002..Lbx1, therefore, plays a critical role in the development of sensory pathways in the spinal cord that relay pain and touch...
- Sonic hedgehog controls epaxial muscle determination through Myf5 activationA G Borycki
Department of Cell Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6058, USA
Development 126:4053-63. 1999....
- Pax3:Foxc2 reciprocal repression in the somite modulates muscular versus vascular cell fate choice in multipotent progenitorsMounia Lagha
CNRS URA 2578, Departement de Biologie du Developpement, Institut Pasteur, 75015 Paris, France
Dev Cell 17:892-9. 2009..to different lineages such as vascular smooth and skeletal muscle, regulated by the transcription factors Foxc2 and Pax3, respectively. Here we show reciprocal inhibition between Pax3 and Foxc2 in the mouse embryo...
- Dmrt2 and Pax3 double-knockout mice show severe defects in embryonic myogenesisKwang Won Seo
Department of Biochemistry and Molecular Biology, MD Anderson Cancer Center, Houston, TX, USA
Comp Med 57:460-8. 2007..Several transcription factors from the dermomyotome affect embryonic myogenesis. Among these, Dmrt2 and Pax3 were tested for genetic and functional interactions during embryonic myogenesis by evaluating myogenin and desmin ..
- A Pax3/Pax7-dependent population of skeletal muscle progenitor cellsFrederic Relaix
C N R S URA 2578, Department of Developmental Biology, Pasteur Institute, 75724 Paris Cedex 15, France
Nature 435:948-53. 2005..We have identified a new cell population that expresses the transcription factors Pax3 and Pax7 (paired box proteins 3 and 7) but no skeletal-muscle-specific markers...
- Pax3 and Pax7 have distinct and overlapping functions in adult muscle progenitor cellsFrederic Relaix
Unité de Génétique Moléculaire du Développement, Centre National de la Recherche Scientifique URA 2578, Departement de Biologie du Developpement
J Cell Biol 172:91-102. 2006..We show that Pax3, the paralogue of Pax7, is also present in both quiescent and activated satellite cells in many skeletal muscles...
- Redefining the genetic hierarchies controlling skeletal myogenesis: Pax-3 and Myf-5 act upstream of MyoDS Tajbakhsh
Department of Molecular Biology, Centre National de la Recherche Scientifique, Unité de Recherche Associe 1947, Pasteur Institute, Paris, France
Cell 89:127-38. 1997We analyzed Pax-3 (splotch), Myf-5 (targeted with nlacZ), and splotch/Myf-5 homozygous mutant mice to investigate the roles that these genes play in programming skeletal myogenesis...
- A novel genetic hierarchy functions during hypaxial myogenesis: Pax3 directly activates Myf5 in muscle progenitor cells in the limbLola Bajard
Centre National de la Recherche Scientifique URA 2578, Department of Developmental Biology, Pasteur Institute, 75015 Paris, France
Genes Dev 20:2450-64. 2006..Null mutations in Pax3, a key regulator of skeletal muscle formation, lead to cell death in this domain...
- Dysgenesis of cephalic neural crest derivatives in Pax7-/- mutant miceA Mansouri
Department of Molecular Cell Biology, Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
Development 122:831-8. 1996..No obvious phenotype could be detected in the central nervous system and skeletal muscle. Functional redundancy between Pax7 and Pax3 is discussed.
- Alveolar rhabdomyosarcomas in conditional Pax3:Fkhr mice: cooperativity of Ink4a/ARF and Trp53 loss of functionCharles Keller
Division of Pediatric Hematology Oncology and Department of Pediatrics, University of Utah, Salt Lake City, Utah 84112, USA
Genes Dev 18:2614-26. 2004..We report the first mouse model of alveolar rhabdomyosarcoma using a conditional Pax3:Fkhr knock-in allele whose activation in late embryogenesis and postnatally is targeted to terminally ..
- Insertion of Cre into the Pax3 locus creates a new allele of Splotch and identifies unexpected Pax3 derivativesKurt A Engleka
Cardiovascular Division, University of Pennsylvania, Philadelphia, 19104, USA
Dev Biol 280:396-406. 2005..Pax3-deficient Splotch embryos display neural tube and neural crest defects and lack hypaxial muscles...
- Pax-3, a novel murine DNA binding protein expressed during early neurogenesisM D Goulding
Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
EMBO J 10:1135-47. 1991..In 10-12 day embryos, expression of Pax-3 is also seen in neural crest cells of the developing spinal ganglia, the craniofacial mesectoderm and in limb mesenchyme of 10 and 11 day embryos...
- Pax3 functions at a nodal point in melanocyte stem cell differentiationDeborah Lang
Cardiovascular Division, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
Nature 433:884-7. 2005..Here we describe the molecular details of a nodal point in adult melanocyte stem cell differentiation in which Pax3 simultaneously functions to initiate a melanogenic cascade while acting downstream to prevent terminal ..
- Adult satellite cells and embryonic muscle progenitors have distinct genetic requirementsChristoph Lepper
Department of Embryology, Carnegie Institution, 3520 San Martin Drive, Baltimore, Maryland 21218, USA
Nature 460:627-31. 2009Myogenic potential, survival and expansion of mammalian muscle progenitors depend on the myogenic determinants Pax3 and Pax7 embryonically, and Pax7 alone perinatally...
- Identification of minimal enhancer elements sufficient for Pax3 expression in neural crest and implication of Tead2 as a regulator of Pax3Rita C Milewski
Cardiovascular Division, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA
Development 131:829-37. 2004b>Pax3 is a transcription factor that is required by pre-migratory neural crest cells, which give rise to the peripheral nervous system, melanocytes, some vascular smooth muscle, and numerous other derivatives [corrected]...
- Regulation of Pax-3 expression in the dermomyotome and its role in muscle developmentM Goulding
Molecular Neurobiology Laboratory, Salk Institute, La Jolla, CA 92037, USA
Development 120:957-71. 1994..gene in the differentiation of cell types derived from the dermomyotome by analyzing the development of muscle in splotch mouse embryos which lack a functional Pax-3 gene...
- Essential role for the c-met receptor in the migration of myogenic precursor cells into the limb budF Bladt
Max Delbruck Centrum fur Molekulare Medizin, Berlin, Germany
Nature 376:768-71. 1995..We have therefore identified a paracrine signalling system that regulates migration of myogenic precursor cells...
- Roles of Pax-genes in developing and adult brain as suggested by expression patternsA Stoykova
Department of Molecular Cell Biology, Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
J Neurosci 14:1395-412. 1994....
- Hox/Pbx and Brn binding sites mediate Pax3 expression in vitro and in vivoSteven C Pruitt
Department of Molecular and Cellular Biology, Roswell Park Cancer Institute, Elm and Carlton Streets, Buffalo, NY 14263, USA
Gene Expr Patterns 4:671-85. 2004b>Pax3 is a paired-homeodomain class transcription factor that serves a role in dorsal-ventral and medial-lateral patterning during vertebrate embryogenesis...
- Transcription factor Gbx2 acts cell-nonautonomously to regulate the formation of lineage-restriction boundaries of the thalamusLi Chen
Department of Genetics and Developmental Biology, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT 06030, USA
Development 136:1317-26. 2009..We propose that, within the developing thalamus, the dynamic and differential expression of Gbx2 may be involved in the specific segregation of thalamic neurons, leading to partition of the thalamus into different nuclei...
- Sim1 and Sim2 expression during chick and mouse limb developmentPascal Coumailleau
CNRS, UMR7622, Biologie Moleculaire et Cellulaire du Developpement, Universite Pierre et Marie Curie, Paris, France
Int J Dev Biol 53:149-57. 2009..In addition, chick and mouse Sim2 gene expression is enhanced in limb ventral muscle masses versus dorsal ventral muscle masses. Our results provide a basis for further functional analysis of the Sim genes in limb muscle formation...
- HIRA, a mammalian homologue of Saccharomyces cerevisiae transcriptional co-repressors, interacts with Pax3P Magnaghi
Molecular Medicine Unit, Institute of Child Health, London, UK
Nat Genet 20:74-7. 1998..Pax3 haploinsufficiency results in the mouse splotch and human Waardenburg syndrome (WSI and WSIII) phenotypes...
- Apoptosis of epaxial myotome in Danforth's short-tail (Sd) mice in somites that form following notochord degenerationA Asakura
Division of Molecular Medicine, Fred Hutchinson Cancer Research Center, 1124 Columbia Street, Seattle, Washington, 98104, USA
Dev Biol 203:276-89. 1998..The apoptosis of epaxial muscle in somites that formed after notochord degeneration is consistent with a role for the notochord in the survival and differentiation of the dorsal myotome...
- Molecular characterization of a deletion encompassing the splotch mutation on mouse chromosome 1D J Epstein
Department of Biology, McGill University, Montreal, Quebec, Canada
Genomics 10:89-93. 1991..assigned to the proximal portion of mouse chromosome 1 to characterize the chromosomal segment deleted in the splotch-retarded (Spr) mouse mutant...
- Positive and negative DNA sequence elements are required to establish the pattern of Pax3 expressionT A Natoli
Roswell Park Cancer Institute, Department of Molecular and Cellular Biology, Buffalo, NY 14263, USA
Development 124:617-26. 1997The transcription start site and DNA sequence elements required for the induction of Pax3 expression in differentiating P19 embryonal carcinoma cells have been localized...
- The role of SF/HGF and c-Met in the development of skeletal muscleS Dietrich
King s College, London Bridge, London SE1 9RT, UK de
Development 126:1621-9. 1999..They lack muscle groups that derive from migratory precursor cells, but display otherwise normal skeletal musculature...
- Pax3 inhibits myogenic differentiation of cultured myoblast cellsJ A Epstein
Division of Cardiology, Brigham and Women s Hospital, Boston, Massachusetts, USA
J Biol Chem 270:11719-22. 1995..Mutations in Pax-3 account for the mouse mutant Splotch which develops without limb musculature...
- Early specification of limb muscle precursor cells by the homeobox gene Lbx1hK Schafer
Institute of Physiological Chemistry, Martin Luther University Halle, 06097 Halle, Hollystr 1, Germany
Nat Genet 23:213-6. 1999..6), Tbx1 (ref. 7), Mox2 (ref. 8), Six1 and Six2 (ref. 9), Pitx2, Pax3 (refs 10,11) and Lbx1h (refs 12,13), are expressed in migrating limb precursor cells...
- Evidence for a myotomal Hox/Myf cascade governing nonautonomous control of rib specification within global vertebral domainsTânia Vinagre
Instituto Gulbenkian de Ciencia, Rua da Quinta Grande 6, 2780 156 Oeiras, Portugal
Dev Cell 18:655-61. 2010..Our findings offer a new perspective of how Hox genes produce global patterns in the axial skeleton and support a redundant nonmyogenic role of Myf5 and Myf6 in rib formation...
- Expression of the met receptor tyrosine kinase in muscle progenitor cells in somites and limbs is absent in Splotch miceX M Yang
Molecular Oncology Group, Royal Victoria Hospital, Montreal, Quebec, Canada
Development 122:2163-71. 1996..b>Splotch-2H and Splotch-delayed mice, which harbor mutations in Pax-3, show major disruptions in early limb muscle ..
- Reduced expression of pax-3 is associated with overexpression of cdc46 in the mouse embryoA L Hill
Department of Medicine, Harvard Medical School, Boston, MA 02215, USA
Dev Genes Evol 208:128-34. 1998..of diabetic mice, which we have previously shown express significantly reduced levels of Pax-3 mRNA, and in Splotch embryos, which carry loss of function Pax-3 alleles...
- Pax3 and Pax7 are expressed in commissural neurons and restrict ventral neuronal identity in the spinal cordA Mansouri
Max Planck Institute for Biophysical Chemistry, Department of Molecular Cell Biology, Am Fassberg 11, 37077, Gottingen, Germany
Mech Dev 78:171-8. 1998b>Pax3 and Pax7 are transcription factors sharing high sequence identity and overlapping patterns of expression in particular in the dorsal spinal cord...
- Msx1 antagonizes the myogenic activity of Pax3 in migrating limb muscle precursorsA J Bendall
Center for Advanced Biotechnology and Medicine, Department of Neuroscience and Cell Biology and Department of Pediatrics, UMDNJ Robert Wood Johnson Medical School, Piscataway, NJ 08854, USA
Development 126:4965-76. 1999..Here we show that in chicken embryos, expression of the Msx1 homeobox gene overlaps with Pax3 in migrating limb muscle precursors, which are committed myoblasts that do not express myogenic differentiation ..
- Oxidant regulation of gene expression and neural tube development: Insights gained from diabetic pregnancy on molecular causes of neural tube defectsT I Chang
Section on Molecular Biology and Complications, Joslin Diabetes Center, One Joslin Place, Boston, MA 02215, USA
Diabetologia 46:538-45. 2003..Here we tested the hypothesis that oxidative stress inhibits expression of Pax-3, thereby providing a molecular basis for neural tube defects induced by diabetic pregnancy...
- The transcriptional activator PAX3-FKHR rescues the defects of Pax3 mutant mice but induces a myogenic gain-of-function phenotype with ligand-independent activation of Met signaling in vivoFrederic Relaix
CNRS URA 2375, Department of Developmental Biology, Pasteur Institute, 75724 Paris Cedex 15, France
Genes Dev 17:2950-65. 2003b>Pax3 is a key transcription factor implicated in development and human disease...
- Signals from the notochord and floor plate regulate the region-specific expression of two Pax genes in the developing spinal cordM D Goulding
Department of Molecular Cell Biology, Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
Development 117:1001-16. 1993..The rapid changes to Pax gene expression that occur in neural progenitor cells following the grafting of an ectopic notochord suggest that changes to Pax gene expression are an early effect of the notochord on spinal cord patterning...
- The Splotch mutation interferes with muscle development in the limbsT Franz
Anatomisches Institut, Abteilung fur Neuroanatomie, Universitats Krankenhaus Eppendorf, Hamburg, Germany
Anat Embryol (Berl) 187:153-60. 1993Homozygosity for the Splotch mutation causes neural tube and neural crest defects in mice. It has been demonstrated that Splotch mutant mice carry mutations in the homeodomain of the Pax-3 gene...
- Mesodermal defects and cranial neural crest apoptosis in alpha5 integrin-null embryosK L Goh
Center for Cancer Research and Department of Biology, Massachusetts Institute of Technology, Cambridge 02139, USA
Development 124:4309-19. 1997....
- Mox2 is a component of the genetic hierarchy controlling limb muscle developmentB S Mankoo
Division of Developmental Neurobiology, MRC National Institute for Medical Research, London, UK
Nature 400:69-73. 1999..encoding the basic helix-loop-helix (bHLH) myogenic regulatory factors (MRFs; MyoD, Myf5, myogenin and MRF4) and Pax3, a paired-type homeobox gene that is critical for the development of limb musculature...
- Pax3 acts cell autonomously in the neural tube and somites by controlling cell surface propertiesA Mansouri
Max Planck Institute for Biophysical Chemistry, Department of Molecular Cell Biology, Am Fassberg 11, D 37077 Gottingen, Germany
Development 128:1995-2005. 2001..Several loss-of-function mutations are correlated with the Splotch phenotype in mice and Waardenburg syndrome in humans...
- Pax3 induces cell aggregation and regulates phenotypic mesenchymal-epithelial interconversionO Neil Wiggan
Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, Ontario, M5S 1A8 Canada
J Cell Sci 115:517-29. 2002..Although mutations to Pax3 and other Pax-family genes in both mice and humans result in numerous tissue-specific morphological defects, little ..
- Rescue of neural tube defects in Pax-3-deficient embryos by p53 loss of function: implications for Pax-3- dependent development and tumorigenesisLydie Pani
Section on Cellular and Molecular Physiology, Joslin Diabetes Center, Harvard Medical School, Boston, Massachusetts 02215, USA
Genes Dev 16:676-80. 2002..We previously showed that apoptosis is associated with neural tube defects (NTDs) in Pax-3-deficient Splotch (Sp/Sp) embryos...
- Divergent functions of murine Pax3 and Pax7 in limb muscle developmentFrederic Relaix
Centre National de la Recherche Scientifique CNRS URA 2578, Department of Developmental Biology, Pasteur Institute, 75724 Paris Cedex 15, France
Genes Dev 18:1088-105. 2004Pax genes encode evolutionarily conserved transcription factors that play critical roles in development. Pax3 and Pax7 constitute one of the four Pax subfamilies...
- CXCR4 and Gab1 cooperate to control the development of migrating muscle progenitor cellsElena Vasyutina
Max Delbruck Center for Molecular Medicine, 13125 Berlin, Germany
Genes Dev 19:2187-98. 2005..Our analysis reveals a role of SDF1/CXCR4 signaling in the development of migrating muscle progenitors and shows that a threshold number of progenitor cells is required to generate muscle of appropriate size...
- RBP-J (Rbpsuh) is essential to maintain muscle progenitor cells and to generate satellite cellsElena Vasyutina
Max Delbruck Center for Molecular Medicine, Robert Rossle Strasse 10, 13125 Berlin, Germany
Proc Natl Acad Sci U S A 104:4443-8. 2007..In addition, satellite cells are not formed late in fetal development in conditional RBP-J mutant mice. We conclude that RBP-J is required in the developing muscle to set aside proliferating progenitors and satellite cells...
- Pax3 regulation of FGF signaling affects the progression of embryonic progenitor cells into the myogenic programMounia Lagha
CNRS URA 2578, Department of Developmental Biology, Pasteur Institute, 75015 Paris, France
Genes Dev 22:1828-37. 2008b>Pax3/7-dependent stem cells play an essential role in skeletal muscle development. We now show that Fgfr4 lies genetically downstream from Pax3 and is a direct target...
- SMD and NMD are competitive pathways that contribute to myogenesis: effects on PAX3 and myogenin mRNAsChenguang Gong
Department of Biochemistry and Biophysics, School of Medicine and Dentistry, University of Rochester, Rochester, New York 14642, USA
Genes Dev 23:54-66. 2009..We discuss the remarkable balance between SMD and the two types of NMD in view of data indicating that PAX3 mRNA is an SMD target whose decay promotes myogenesis whereas myogenin mRNA is a classical NMD target encoding a ..
- Waardenburg's syndrome patients have mutations in the human homologue of the Pax-3 paired box geneM Tassabehji
University Department of Medical Genetics, St Mary s Hospital, Manchester, UK
Nature 355:635-6. 1992..linkage to an alkaline phosphatase gene, and their related phenotype, we suggested that WS and the mouse mutant Splotch might be homologous. Splotch is caused by mutation in the mouse Pax-3 gene...
- Splotch (Sp2H), a mutation affecting development of the mouse neural tube, shows a deletion within the paired homeodomain of Pax-3D J Epstein
Department of Biology, McGill University, Montreal, Canada
Cell 67:767-74. 1991The molecular basis of the mouse mutation splotch (Sp), which is associated with spina bifida and exencephaly, was analyzed at three of its alleles, Sp, Sp2H, and Spr...
- Persistent truncus arteriosus in the Splotch mutant mouseT Franz
Anatomisches Institut, Universitäts Krankenhans Eppendorf, Hamburg, Federal Republic of Germany
Anat Embryol (Berl) 180:457-64. 1989The Splotch mutant mouse shows defects in neural crest-derived cell populations...
- Pax3: a paired domain gene as a regulator in PNS myelinationC Kioussi
Abteilung für Molekulare Zellbiologie, Max Planck Institut fur biophysikalische Chemie, Gottingen, Federal Republic of Germany
Neuron 15:553-62. 1995b>Pax3 RNA is expressed in neural crest when Schwann cell (SC) precursors migrate to the PNS. Pax3 RNA and SC markers were monitored in sciatic nerves of mice during development and nerve repair...
- Pax-3 contains domains for transcription activation and transcription inhibitionG Chalepakis
Department of Molecular Cell Biology, Max Planck Institute for Biophysical Chemistry, Gottingen, Federal Republic of Germany
Proc Natl Acad Sci U S A 91:12745-9. 1994..Pax-3 can utilize both transcription modulating functions and activates transcription over a narrow range of protein concentration in the presence of promoter elements containing functional binding sites...
- HNF-3 beta is essential for node and notochord formation in mouse developmentS L Ang
Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
Cell 78:561-74. 1994..HNF-3 beta is not required for the development of definitive endoderm cells, but foregut morphogenesis is severely affected in HNF-3 beta -/- embryos...
- The oncogenic potential of Pax genesC C Maulbecker
Department of Molecular Cell Biology, Max Planck Institute of Biophysical Chemistry, Gottingen, Germany
EMBO J 12:2361-7. 1993..Therefore, the Pax gene products are not only involved in controlling embryogenesis, but they can, if deregulated, also induce tumorigenesis...
- Pax-3 is required for the development of limb muscles: a possible role for the migration of dermomyotomal muscle progenitor cellsE Bober
Department of Cell and Molecular Biology, Technical University of Braunschweig, Germany
Development 120:603-12. 1994..Significantly, in homozygous splotch mutant mice, which synthesize altered Pax-3 mRNA but make no normal protein, no cells positive for Pax-3 ..
- Wnt-3a regulates somite and tailbud formation in the mouse embryoS Takada
Roche Institute of Molecular Biology, Roche Research Center, Nutley, New Jersey 07110
Genes Dev 8:174-89. 1994..We suggest that dysmorphology is secondary to the mesodermal and axial defects and that dorsal patterning of the CNS may be regulated by inductive signals arising from surface ectoderm...
- Analysis of the Pax-3 gene in the mouse mutant splotchM Goulding
Department of Molecular Cell Biology, Max Planck Institute for Biophysical Chemistry, Gottingen, Germany
Genomics 17:355-63. 1993In a linkage analysis of Pax-3 and splotch no recombinations were found in 117 backcross mice. Molecular analysis of Pax-3 in three alleles of splotch shows a number of significant alterations to the Pax-3 gene...
- Requirement for LIM homeobox gene Isl1 in motor neuron generation reveals a motor neuron-dependent step in interneuron differentiationS L Pfaff
Howard Hughes Medical Institute, Department of Biochemistry and Molecule Biophysics, Columbia University, New York, New York 10032, USA
Cell 84:309-20. 1996..These results show that ISL1 is required for the generation of motor neurons and suggest that motor neuron generation is required for the subsequent differentiation of certain interneurons...
- Pax3 modulates expression of the c-Met receptor during limb muscle developmentJ A Epstein
Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, MA, USA
Proc Natl Acad Sci U S A 93:4213-8. 1996..Here, we show that c-met expression is markedly reduced in the lateral dermomyotome of Splotch embryos lacking Pax3...
- Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene functionC Chiang
Laboratory of Mammalian Genes and Development, National Institute of Health, Bethesda, Maryland 20892, USA
Nature 383:407-13. 1996..Defects in all tissues extend beyond the normal sites of Shh transcription, confirming the proposed role of Shh proteins as an extracellular signal required for the tissue-organizing properties of several vertebrate patterning centres...
- Pax3 is required for cardiac neural crest migration in the mouse: evidence from the splotch (Sp2H) mutantS J Conway
Division of Cell and Molecular Biology, Institute of Child Health, University of London, UK
Development 124:505-14. 1997..Mutation of Pax3, as occurs in the splotch (Sp2H) mouse, results in development of conotruncal heart defects including persistent truncus arteriosus...
- Ectopic Pax-3 activates MyoD and Myf-5 expression in embryonic mesoderm and neural tissueM Maroto
Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, Massachusetts 02115, USA
Cell 89:139-48. 1997..Together, these findings imply that Pax-3 may mediate activation of MyoD and Myf-5 in response to muscle-inducing signals from either the axial tissues or overlying ectoderm and identify Pax-3 as a key regulator of somitic myogenesis...
- Neural tube defects in embryos of diabetic mice: role of the Pax-3 gene and apoptosisS A Phelan
Section on Molecular Biology, Joslin Diabetes Center, Harvard Medical School, Boston, Massachusetts 02215, USA
Diabetes 46:1189-97. 1997..This pathway may be responsible for many of the neural tube defects resulting from diabetic pregnancy...
- Pax6 controls progenitor cell identity and neuronal fate in response to graded Shh signalingJ Ericson
Howard Hughes Medical Institute, Department of Biochemistry and Molecular Biophysics, Columbia University, New York, New York 10032, USA
Cell 90:169-80. 1997..2. Pax6 establishes distinct ventral progenitor cell populations and controls the identity of motor neurons and ventral interneurons, mediating graded Shh signaling in the ventral spinal cord and hindbrain...
- Interaction of the pRB-family proteins with factors containing paired-like homeodomainsO Wiggan
Department of Pathology, University of Toronto, Ontario, Canada
Oncogene 16:227-36. 1998..These data demonstrate that the pRB-family proteins can modulate the activity of factors which specifically control cell fate and/or differentiation as well as controlling cell cycle regulators...
- Three neural tubes in mouse embryos with mutations in the T-box gene Tbx6D L Chapman
Department of Genetics and Development, College of Physicians and Surgeons of Columbia University, New York, New York 10032, USA
Nature 391:695-7. 1998....
- Neurulation abnormalities secondary to altered gene expression in neural tube defect susceptible Splotch embryosG D Bennett
Department of Veterinary Anatomy and Public Health, Texas A and M University, College Station 77843, USA
Teratology 57:17-29. 1998The murine mutant Splotch (Sp) is a well-established model for studying neural tube closure defects...
- Pax-3 is necessary but not sufficient for lbx1 expression in myogenic precursor cells of the limbD Mennerich
Department of Cell and Molecular Biology, University of Braunschweig, Spielmannstrasse 7, 38106, Braunschweig, Germany
Mech Dev 73:147-58. 1998..Analysis of splotch mutant mice showed that lbx1 and c-met are differently affected by the lack of Pax-3...
- Identification of Dep-1, a new gene regulated by the transcription factor Pax-3, as a marker for altered embryonic gene expression during diabetic pregnancyJ Cai
Joslin Diabetes Center, and Department of Medicine, Harvard Medical School, Boston, Massachusetts 02215, USA
Diabetes 47:1803-5. 1998
- Pax3 functions in cell survival and in pax7 regulationA G Borycki
Department of Cell and Developmental Biology, Division of Cardiovascular Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
Development 126:1665-74. 1999..and gene expression in presomitic mesoderm, somites and neural tube of developing wild-type and Pax3 mutant (Splotch) mouse embryos...
- Transgenic rescue of congenital heart disease and spina bifida in Splotch miceJ Li
Cardiovascular Division, Department of Medicine, Department of Cell and Developmental Biology and the Pennsylvania Muscle Institute, University of Pennsylvania, Philadelphia, PA 19104, USA
Development 126:2495-503. 1999Pax3-deficient Splotch mice display neural tube defects and an array of neural crest related abnormalities including defects in the cardiac outflow tract, dorsal root ganglia and pigmentation...
- PAX3 gene structure, alternative splicing and evolutionT D Barber
National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, MD 20850, USA
Gene 237:311-9. 1999..syndrome (types 1 and 3), Craniofacial-deafness-hand syndrome and alveolar rhabdomyosarcoma in humans and the Splotch phenotype in mice...
- Lbx1 is required for muscle precursor migration along a lateral pathway into the limbM K Gross
Molecular Neurobiology Laboratory, The Salk Institute, La Jolla, CA 92037, USA
Development 127:413-24. 2000..Previous studies have shown that Pax3, together with the c-Met receptor tyrosine kinase and its ligand Scatter Factor (SF) are necessary for the ..
- The role of Lbx1 in migration of muscle precursor cellsH Brohmann
Max Delbruck Centre for Molecular Medicine, Robert Rossle Strasse 10, Germany
Development 127:437-45. 2000..We suggest that Lbx1 controls the expression of genes that are essential for the recognition or interpretation of cues that guide migrating muscle precursors and maintain their migratory potential...
- Migration of cardiac neural crest cells in Splotch embryosJ A Epstein
Cardiovascular Division, Department of Medicine and the Department of Cell and Molecular Biology, University of Pennsylvania Medical School, Philadelphia, PA 19104, USA
Development 127:1869-78. 2000..In the absence of Pax3, both humans and mice develop with neural crest defects. Homozygous Splotch embryos that lack Pax3 die by embryonic day 13...
- Homeodomain proteins Mox1 and Mox2 associate with Pax1 and Pax3 transcription factorsD Stamataki
University of Crete Medical School, Heraklion, Crete, Greece
FEBS Lett 499:274-8. 2001..Pax1 and Pax3 gene products are also implicated in these processes...
- Msx2 is an immediate downstream effector of Pax3 in the development of the murine cardiac neural crestStanford J Kwang
Center for Craniofacial Molecular Biology, School of Dentistry, University of Southern California, 1441 Eastlake Avenue, Los Angeles, CA 90089 9176, USA
Development 129:527-38. 2002..In Splotch mutant mice, which lack a functional Pax3 gene, migration of cardiac neural crest is deficient and ..
- Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcomaThomas D Barber
National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, Maryland 20850, USA
Genomics 79:278-84. 2002b>PAX3 is a transcription factor important for neural, muscle, and facial development in vertebrates...
- Identification of a Wnt/Dvl/beta-Catenin --> Pitx2 pathway mediating cell-type-specific proliferation during developmentChrissa Kioussi
Howard Hughes Medical Institute, University of California, San Diego, School of Medicine, CMM West, Room 345, 9500 Gilman Drive, La Jolla, CA 92093, USA
Cell 111:673-85. 2002....
- The homeobox gene Lbx1 specifies a subpopulation of cardiac neural crest necessary for normal heart developmentKonstanze Schäfer
Institute of Physiological Chemistry, University of Halle Wittenberg, Halle, Germany
Circ Res 92:73-80. 2003..activity of the Lbx1 promoter, as indicated by a LacZ reporter gene, is upregulated in the hearts of Lbx1(+/-):splotch(1H)/splotch(1H) and Lbx1(-/-) mice, indicating that Pax3 and Lbx1 participate in a negative regulatory feedback ..
- Alternative splicing of Pax3 produces a transcriptionally inactive proteinColin Pritchard
Department of Genetics, St Jude Children s Research Hospital, 332 North Lauderdale, Memphis, TN 38105, USA
Gene 305:61-9. 2003b>Pax3, a member of the paired-class homeodomain family of transcription factors, plays an important role in embryonic development of neurepithelium and mesenchyme-derived tissues in the mouse and is an early marker for myogenic ..
- Sox10 and Pax3 physically interact to mediate activation of a conserved c-RET enhancerDeborah Lang
Cardiovascular Division, Department of Medicine, University of Pennsylvania, 421 Curie Boulevard, Philadelphia, PA 19104, USA
Hum Mol Genet 12:937-45. 2003..One example is Waardenburg syndrome that can be caused by mutations in the PAX3, SOX10 or MITF genes...
- Analysis of Mlc-lacZ Met mutants highlights the essential function of Met for migratory precursors of hypaxial muscles and reveals a role for Met in the development of hyoid arch-derived facial musclesChiara Prunotto
Department of Anatomy, Pharmacology and Forensic Medicine, University of Torino, Corso Massimo d Azeglio 52, 10126 Turin, Italy
Dev Dyn 231:582-91. 2004The Pax3 and c-met genes are necessary for the development of tongue, diaphragm, and limb muscles...
- Development of the inner ear in Splotch mutant miceDaniela Buckiova
Institute of Experimental Medicine, Academy of Sciences of the Czech Republic, Videnska 1083, 142 20 Prague 4, Czech Republic
Neuroreport 15:2001-5. 2004The Splotch mouse, a Pax 3 mutation, represents a model of Waardenburg syndrome I...
- Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of originCharles Keller
Division of Pediatric Hematology Oncology, Department of Pediatrics, University of Utah, Salt Lake City, Utah 84112, USA
Genes Dev 18:2608-13. 2004To investigate the role of the translocation-associated gene Pax3:Fkhr in alveolar rhabdomyosarcomas, we generated a Cre-mediated conditional knock-in of Pax3:Fkhr into the mouse Pax3 locus...
- Pax3/Pax7 mark a novel population of primitive myogenic cells during developmentLina Kassar-Duchossoy
Department of Developmental Biology, Pasteur Institute, Centre Nationale de la Recherche Scientifique Unité de Recherche Associeé 2578, 75724 Paris, Cedex 15, France
Genes Dev 19:1426-31. 2005..We identify a novel population of resident Pax3+/Pax7+, muscle marker-negative cells throughout development...
- Targeted disruption of the DM domain containing transcription factor Dmrt2 reveals an essential role in somite patterningKwang Won Seo
Department of Biochemistry and Molecular Biology, University of Texas, MD Anderson Cancer Center, Houston, 77030, USA
Dev Biol 290:200-10. 2006..defects are alterations in the expression patterns of dermomyotomal and myotomal transcription factors including Pax3, Paraxis, Myf5, myogenin, Mrf4 and MyoD. Despite these defects, embryos harvested from E13...
- Regulation of murine TGFbeta2 by Pax3 during early embryonic developmentChandra S K Mayanil
Laboratory of Neural Tube Research, Department of Pediatric Neurosurgery, Children s Memorial Research Center and Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60614, USA
J Biol Chem 281:24544-52. 2006Previously our laboratory identified TGFbeta2 as a potential downstream target of Pax3 by utilizing microarray analysis and promoter data base mining (Mayanil, C. S. K., George, D., Freilich, L., Miljan, E. J., Mania-Farnell, B. J...
- Premature myogenic differentiation and depletion of progenitor cells cause severe muscle hypotrophy in Delta1 mutantsKarin Schuster-Gossler
Institute for Molecular Biology, Medizinische Hochschule Hannover, Carl Neuberg Strasse 1, Hannover, D 30625 Lower Saxony, Germany
Proc Natl Acad Sci U S A 104:537-42. 2007..Resident progenitor cells defined by Pax3/Pax7 expression are formed initially, but they are progressively lost and virtually absent at embryonic day 14.5...
- Sequential expression and redundancy of Pitx2 and Pitx3 genes during muscle developmentAurore L'honoré
Laboratoire de Genetique Moleculaire, Institut de recherches cliniques de Montreal IRCM, 110, avenue des Pins Ouest, Montreal, QC, Canada H2W 1R7
Dev Biol 307:421-33. 2007..factors acting during muscle development, including bHLH muscle regulatory factors (MRFs), the paired factors Pax3 and Pax7 and the homeobox factors Six1 and Six4...
- Regulation of Pax3 by proteasomal degradation of monoubiquitinated protein in skeletal muscle progenitorsStéphane C Boutet
Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA
Cell 130:349-62. 2007b>Pax3 and Pax7 play distinct but overlapping roles in developmental and postnatal myogenesis. The mechanisms involved in the differential regulation of these highly homologous proteins are unknown...
- Key basic helix-loop-helix transcription factor genes Hes1 and Ngn2 are regulated by Pax3 during mouse embryonic developmentHiromichi Nakazaki
Laboratory for Neural Tube Research, Developmental Biology Program, Children s Memorial Research Center, Northwestern University Feinberg School of Medicine, Chicago, IL 60614, USA
Dev Biol 316:510-23. 2008..In this study we found that in the absence of functional Pax3, as in Splotch embryos, the neural crest cells undergo premature neurogenesis, as evidenced by increased Brn3a positive staining ..
- Cardiac outflow tract septation failure in Pax3-deficient embryos is due to p53-dependent regulation of migrating cardiac neural crestSarah C Morgan
Section on Developmental and Stem Cell Biology, Joslin Diabetes Center, One Joslin Place, Boston, MA 02215, USA
Mech Dev 125:757-67. 2008During neural tube closure, Pax3 is required to inhibit p53-dependent apoptosis...
- Codependent activators direct myoblast-specific MyoD transcriptionPing Hu
Howard Hughes Medical Institute, Department of Molecular and Cell Biology, University of California, Berkeley, Berkeley, CA 94720, USA
Dev Cell 15:534-46. 2008..Here, we identify MyoD, a key regulator of myogenesis, as a direct target of FoxO3 and Pax3/7 in myoblasts...
- Integrated functions of Pax3 and Pax7 in the regulation of proliferation, cell size and myogenic differentiationCharlotte A Collins
Dubowitz Neuromuscular Centre, UCL Institute of Child Health, London, United Kingdom
PLoS ONE 4:e4475. 2009b>Pax3 and Pax7 are paired-box transcription factors with roles in developmental and adult regenerative myogenesis...
- Credentialing a preclinical mouse model of alveolar rhabdomyosarcomaKoichi Nishijo
Greehey Children s Cancer Research Institute, University of Texas Health Science Center, San Antonio, Texas, USA
Cancer Res 69:2902-11. 2009..e., activation of Pax3:Fkhr fusion gene with either p53 or Cdkn2a inactivation...
- Id3 is a direct transcriptional target of Pax7 in quiescent satellite cellsDeepak Kumar
Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA 02115, USA
Mol Biol Cell 20:3170-7. 2009Pax7 is a key regulator of skeletal muscle stem cells and is required along with Pax3 to generate skeletal muscle precursors. We have identified a collection of genes induced by either Pax3 or Pax7 in C2C12 muscle cells...
- Muscle contraction is necessary to maintain joint progenitor cell fateJoy Kahn
Department of Molecular Genetics, Weizmann Institute of Science, Rehovot, Israel
Dev Cell 16:734-43. 2009..In conclusion, our findings provide the missing link between progenitor cell fate determination and embryonic movement, two processes shown to be essential for correct organogenesis...
- Muscle stem cell behavior is modified by microRNA-27 regulation of Pax3 expressionColin G Crist
Centre National de Recherche Scientifique Unité de Recherche Associée 2578, Department of Developmental Biology, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
Proc Natl Acad Sci U S A 106:13383-7. 2009Skeletal muscle stem cells are regulated by Pax3/7...
- Distinct enhancers at the Pax3 locus can function redundantly to regulate neural tube and neural crest expressionsKarl R Degenhardt
Division of Cardiology, Department of Pediatrics, Children s Hospital of Philadelphia, PA, USA
Dev Biol 339:519-27. 2010b>Pax3 is a transcription factor expressed in somitic mesoderm, dorsal neural tube and pre-migratory neural crest during embryonic development...
- A Pax3/Dmrt2/Myf5 regulatory cascade functions at the onset of myogenesisTakahiko Sato
Department of Developmental Biology, Centre National de la Recherche Scientifique, Unité de Recherche Associée 2578, Institut Pasteur, Paris, France
PLoS Genet 6:e1000897. 2010..These multipotent stem cells express Pax3, and this expression is maintained in the myogenic lineage where Pax3 plays an important role...
- INVESTIGATING THE ROLE OF CDO IN BRAIN DEVELOPMENTAmi Okada; Fiscal Year: 2004..These experiments should also contribute to the overall understanding of the signals that are important in the assembly of the nervous system. ..
- Mice That Lack CDO: A Model for Mild HoloprosencephalyROBERT KRAUSS; Fiscal Year: 2004....
- Functional Analysis of Spinal Cord InterneuronsMartyn Goulding; Fiscal Year: 2005..These studies will provide valuable insights into the development and organization of circuits in the spinal cord that control locomotor behavior, and will lay the groundwork for strategies to treat spinal cord injury and degeneration. ..
- 2005 NNFF Consortium for NF1, NF2 and SchwannomatosisJonathan Epstein; Fiscal Year: 2005....
- Specification of Dorsal Lbx1-Derived InterneuronsMartyn Goulding; Fiscal Year: 2007..These studies will provide important insights into the development and organization of neural circuits in the dorsal horn that sense and relay pain. [unreadable] [unreadable] [unreadable]..
- Comprehensive Protein Interaction Mapping in MammalsSTEVEN PRUITT; Fiscal Year: 2004..Based on the utility of similar protein interaction maps in model organisms, this work can be expected to provide an enormous wealth of insight into the function of mammalian cells. ..
- Mechanisms of Nural Crest Cell DevelopmentChing Pin Chang; Fiscal Year: 2010..Aim #3: Define the molecular pathways affected by the absence of Pbx1. We will use standard molecular biology and embryology methods to study if Pax3 pathway is downstream of Pbx1 in cardiac development.
- Molecular basis of vascular heterogenity and functionJonathan Epstein; Fiscal Year: 2008..abstract_text> ..
- Neural Stem Cell Survival and Function in Aging Mice an*STEVEN PRUITT; Fiscal Year: 2004..The ability to monitor the fates of neural stem cell progeny in vivo will allow us to determine if age- related changes in the differentiation potentials of these cells occur. ..
- GENETIC ANALYSIS OF PAX3 INDUCTIONSTEVEN PRUITT; Fiscal Year: 2003..We have developed a unique set of reagents that make analysis of expression from the Pax3 gene in the mouse a particularly tractable system for understanding how DV and AP positional information is ..
- Development of a high throughput gene trap vetorSTEVEN PRUITT; Fiscal Year: 2003..The methodologies and reagents developed here will be fully sufficient to allow the rapid establishment of a random sequence tag insertion library of 100,000 or more events in which the location of each insertion sites is known. ..
- RAS MEDIATED, ANCHORAGE INDEPENDENT GROWTHROBERT KRAUSS; Fiscal Year: 2002..Such genes represent potential mediators of the transformed phenotype. ..
- MOLECULAR ANALYSIS OF MYOTONIC DYSTROPHYSATYAKAM BHAGWATI; Fiscal Year: 2002..This approach is designed to explore the function of these CTG/CUG repeat binding proteins and their possible role in the pathogenesis of DM. ..
- Transcription Factor P53 in Terminal Nephron DifferentZubaida Saifudeen; Fiscal Year: 2004..The results of this work will provide information on what determines terminal renal differentiation. In addition, these data will advance our understanding of the role the tumor suppressor p53 in kidney development...
- Role of CD164 in Skeletal MyogenesisROBERT KRAUSS; Fiscal Year: 2008..This research should therefore shed light on fundamental processes by which skeletal muscles develop. [unreadable] [unreadable]..
- Gene-Environment Interaction in Holoprosencephaly: Role of Fetal Alcohol ExposureROBERT KRAUSS; Fiscal Year: 2008..Such information may have important public health impact and could ultimately aid in the counseling of HPE mutation carriers. [unreadable] [unreadable] [unreadable]..
- LBX2 AND UROGENITAL DEVELOPMENTJonathan Epstein; Fiscal Year: 2002....
- PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENTJonathan Epstein; Fiscal Year: 2008unreadable] DESCRIPTION (provided by applicant): This grant application focuses on the regulation and function of Pax3 in neural crest ceils...